Acute liver failure in children: The first 348 patients in the pediatric acute liver failure study group

Robert H. Squires, Benjamin L. Shneider, John Bucuvalas, Estella Alonso, Ronald J. Sokol, Michael R. Narkewicz, Anil Dhawan, Philip Rosenthal, Norberto Rodriguez-Baez, Karen F. Murray, Simon Horslen, Martin G. Martin, M. James Lopez, Humberto Soriano, Brendan M. McGuire, Maureen M. Jonas, Nada Yazigi, Ross W. Shepherd, Kathleen Schwarz, Steven LobrittoDaniel W. Thomas, Joel E. Lavine, Saul Karpen, Vicky Ng, Deirdre Kelly, Nancy Simonds, Linda S. Hynan

Research output: Contribution to journalArticle

414 Citations (Scopus)

Abstract

Objectives: To determine short-term outcome for children with acute liver failure (ALF) as it relates to cause, clinical status, and patient demographics and to determine prognostic factors. Study design: A prospective, multicenter case study collecting demographic, clinical, laboratory, and short-term outcome data on children from birth to 18 years with ALF. Patients without encephalopathy were included if the prothrombin time and international normalized ratio remained ≥20 seconds and/or >2, respectively, despite vitamin K. Primary outcome measures 3 weeks after study entry were death, death after transplantation, alive with native liver, and alive with transplanted organ. Results: The cause of ALF in 348 children included acute acetaminophen toxicity (14%), metabolic disease (10%), autoimmune liver disease (6%), non-acetaminophen drug-related hepatotoxicity (5%), infections (6%), other diagnosed conditions (10%); 49% were indeterminate. Outcome varied between patient sub-groups; 20% with non-acetaminophen ALF died or underwent liver transplantation and never had clinical encephalopathy. Conclusions: Causes of ALF in children differ from in adults. Clinical encephalopathy may not be present in children. The high percentage of indeterminate cases provides an opportunity for investigation.

Original languageEnglish (US)
JournalJournal of Pediatrics
Volume148
Issue number5
DOIs
StatePublished - May 2006

Fingerprint

Acute Liver Failure
Pediatrics
Brain Diseases
Demography
International Normalized Ratio
Vitamin K
Prothrombin Time
Metabolic Diseases
Acetaminophen
Liver Transplantation
Autoimmune Diseases
Multicenter Studies
Liver Diseases
Transplantation
Outcome Assessment (Health Care)
Parturition
Liver
Infection
Pharmaceutical Preparations

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Acute liver failure in children : The first 348 patients in the pediatric acute liver failure study group. / Squires, Robert H.; Shneider, Benjamin L.; Bucuvalas, John; Alonso, Estella; Sokol, Ronald J.; Narkewicz, Michael R.; Dhawan, Anil; Rosenthal, Philip; Rodriguez-Baez, Norberto; Murray, Karen F.; Horslen, Simon; Martin, Martin G.; Lopez, M. James; Soriano, Humberto; McGuire, Brendan M.; Jonas, Maureen M.; Yazigi, Nada; Shepherd, Ross W.; Schwarz, Kathleen; Lobritto, Steven; Thomas, Daniel W.; Lavine, Joel E.; Karpen, Saul; Ng, Vicky; Kelly, Deirdre; Simonds, Nancy; Hynan, Linda S.

In: Journal of Pediatrics, Vol. 148, No. 5, 05.2006.

Research output: Contribution to journalArticle

Squires, RH, Shneider, BL, Bucuvalas, J, Alonso, E, Sokol, RJ, Narkewicz, MR, Dhawan, A, Rosenthal, P, Rodriguez-Baez, N, Murray, KF, Horslen, S, Martin, MG, Lopez, MJ, Soriano, H, McGuire, BM, Jonas, MM, Yazigi, N, Shepherd, RW, Schwarz, K, Lobritto, S, Thomas, DW, Lavine, JE, Karpen, S, Ng, V, Kelly, D, Simonds, N & Hynan, LS 2006, 'Acute liver failure in children: The first 348 patients in the pediatric acute liver failure study group', Journal of Pediatrics, vol. 148, no. 5. https://doi.org/10.1016/j.jpeds.2005.12.051
Squires, Robert H. ; Shneider, Benjamin L. ; Bucuvalas, John ; Alonso, Estella ; Sokol, Ronald J. ; Narkewicz, Michael R. ; Dhawan, Anil ; Rosenthal, Philip ; Rodriguez-Baez, Norberto ; Murray, Karen F. ; Horslen, Simon ; Martin, Martin G. ; Lopez, M. James ; Soriano, Humberto ; McGuire, Brendan M. ; Jonas, Maureen M. ; Yazigi, Nada ; Shepherd, Ross W. ; Schwarz, Kathleen ; Lobritto, Steven ; Thomas, Daniel W. ; Lavine, Joel E. ; Karpen, Saul ; Ng, Vicky ; Kelly, Deirdre ; Simonds, Nancy ; Hynan, Linda S. / Acute liver failure in children : The first 348 patients in the pediatric acute liver failure study group. In: Journal of Pediatrics. 2006 ; Vol. 148, No. 5.
@article{09fceeda93f048ca8da1bacca781a15e,
title = "Acute liver failure in children: The first 348 patients in the pediatric acute liver failure study group",
abstract = "Objectives: To determine short-term outcome for children with acute liver failure (ALF) as it relates to cause, clinical status, and patient demographics and to determine prognostic factors. Study design: A prospective, multicenter case study collecting demographic, clinical, laboratory, and short-term outcome data on children from birth to 18 years with ALF. Patients without encephalopathy were included if the prothrombin time and international normalized ratio remained ≥20 seconds and/or >2, respectively, despite vitamin K. Primary outcome measures 3 weeks after study entry were death, death after transplantation, alive with native liver, and alive with transplanted organ. Results: The cause of ALF in 348 children included acute acetaminophen toxicity (14{\%}), metabolic disease (10{\%}), autoimmune liver disease (6{\%}), non-acetaminophen drug-related hepatotoxicity (5{\%}), infections (6{\%}), other diagnosed conditions (10{\%}); 49{\%} were indeterminate. Outcome varied between patient sub-groups; 20{\%} with non-acetaminophen ALF died or underwent liver transplantation and never had clinical encephalopathy. Conclusions: Causes of ALF in children differ from in adults. Clinical encephalopathy may not be present in children. The high percentage of indeterminate cases provides an opportunity for investigation.",
author = "Squires, {Robert H.} and Shneider, {Benjamin L.} and John Bucuvalas and Estella Alonso and Sokol, {Ronald J.} and Narkewicz, {Michael R.} and Anil Dhawan and Philip Rosenthal and Norberto Rodriguez-Baez and Murray, {Karen F.} and Simon Horslen and Martin, {Martin G.} and Lopez, {M. James} and Humberto Soriano and McGuire, {Brendan M.} and Jonas, {Maureen M.} and Nada Yazigi and Shepherd, {Ross W.} and Kathleen Schwarz and Steven Lobritto and Thomas, {Daniel W.} and Lavine, {Joel E.} and Saul Karpen and Vicky Ng and Deirdre Kelly and Nancy Simonds and Hynan, {Linda S.}",
year = "2006",
month = "5",
doi = "10.1016/j.jpeds.2005.12.051",
language = "English (US)",
volume = "148",
journal = "Journal of Pediatrics",
issn = "0022-3476",
publisher = "Mosby Inc.",
number = "5",

}

TY - JOUR

T1 - Acute liver failure in children

T2 - The first 348 patients in the pediatric acute liver failure study group

AU - Squires, Robert H.

AU - Shneider, Benjamin L.

AU - Bucuvalas, John

AU - Alonso, Estella

AU - Sokol, Ronald J.

AU - Narkewicz, Michael R.

AU - Dhawan, Anil

AU - Rosenthal, Philip

AU - Rodriguez-Baez, Norberto

AU - Murray, Karen F.

AU - Horslen, Simon

AU - Martin, Martin G.

AU - Lopez, M. James

AU - Soriano, Humberto

AU - McGuire, Brendan M.

AU - Jonas, Maureen M.

AU - Yazigi, Nada

AU - Shepherd, Ross W.

AU - Schwarz, Kathleen

AU - Lobritto, Steven

AU - Thomas, Daniel W.

AU - Lavine, Joel E.

AU - Karpen, Saul

AU - Ng, Vicky

AU - Kelly, Deirdre

AU - Simonds, Nancy

AU - Hynan, Linda S.

PY - 2006/5

Y1 - 2006/5

N2 - Objectives: To determine short-term outcome for children with acute liver failure (ALF) as it relates to cause, clinical status, and patient demographics and to determine prognostic factors. Study design: A prospective, multicenter case study collecting demographic, clinical, laboratory, and short-term outcome data on children from birth to 18 years with ALF. Patients without encephalopathy were included if the prothrombin time and international normalized ratio remained ≥20 seconds and/or >2, respectively, despite vitamin K. Primary outcome measures 3 weeks after study entry were death, death after transplantation, alive with native liver, and alive with transplanted organ. Results: The cause of ALF in 348 children included acute acetaminophen toxicity (14%), metabolic disease (10%), autoimmune liver disease (6%), non-acetaminophen drug-related hepatotoxicity (5%), infections (6%), other diagnosed conditions (10%); 49% were indeterminate. Outcome varied between patient sub-groups; 20% with non-acetaminophen ALF died or underwent liver transplantation and never had clinical encephalopathy. Conclusions: Causes of ALF in children differ from in adults. Clinical encephalopathy may not be present in children. The high percentage of indeterminate cases provides an opportunity for investigation.

AB - Objectives: To determine short-term outcome for children with acute liver failure (ALF) as it relates to cause, clinical status, and patient demographics and to determine prognostic factors. Study design: A prospective, multicenter case study collecting demographic, clinical, laboratory, and short-term outcome data on children from birth to 18 years with ALF. Patients without encephalopathy were included if the prothrombin time and international normalized ratio remained ≥20 seconds and/or >2, respectively, despite vitamin K. Primary outcome measures 3 weeks after study entry were death, death after transplantation, alive with native liver, and alive with transplanted organ. Results: The cause of ALF in 348 children included acute acetaminophen toxicity (14%), metabolic disease (10%), autoimmune liver disease (6%), non-acetaminophen drug-related hepatotoxicity (5%), infections (6%), other diagnosed conditions (10%); 49% were indeterminate. Outcome varied between patient sub-groups; 20% with non-acetaminophen ALF died or underwent liver transplantation and never had clinical encephalopathy. Conclusions: Causes of ALF in children differ from in adults. Clinical encephalopathy may not be present in children. The high percentage of indeterminate cases provides an opportunity for investigation.

UR - http://www.scopus.com/inward/record.url?scp=33646946176&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=33646946176&partnerID=8YFLogxK

U2 - 10.1016/j.jpeds.2005.12.051

DO - 10.1016/j.jpeds.2005.12.051

M3 - Article

C2 - 16737880

AN - SCOPUS:33646946176

VL - 148

JO - Journal of Pediatrics

JF - Journal of Pediatrics

SN - 0022-3476

IS - 5

ER -